Please use this identifier to cite or link to this item: http://repositorio.unifesp.br/handle/11600/34949
Title: Cerebellar Cognitive Affective Syndrome in Machado Joseph Disease: Core Clinical Features
Authors: Braga-Neto, Pedro [UNIFESP]
Pedroso, Jose Luiz [UNIFESP]
Alessi, Helena [UNIFESP]
Dutra, Livia Almeida [UNIFESP]
Felicio, Andre Carvalho [UNIFESP]
Minett, Thais [UNIFESP]
Weisman, Patricia [UNIFESP]
Santos-Galduroz, Ruth F.
Bertolucci, Paulo Henrique F. [UNIFESP]
Gabbai, Alberto Alain [UNIFESP]
Barsottini, Orlando Graziani Povoas [UNIFESP]
Universidade Federal de São Paulo (UNIFESP)
Hosp Israelita Albert Einstein
Univ Cambridge
Universidade Federal do ABC (UFABC)
UNESP
Keywords: Spinocerebellar ataxia type 3
Machado-Joseph disease
Cognitive deficits
Cerebellar Cognitive Affective Syndrome
Issue Date: 1-Jun-2012
Publisher: Springer
Citation: Cerebellum. New York: Springer, v. 11, n. 2, p. 549-556, 2012.
Abstract: The cerebellum is no longer considered a purely motor control device, and convincing evidence has demonstrated its relationship to cognitive and emotional neural circuits. the aims of the present study were to establish the core cognitive features in our patient population and to determine the presence of Cerebellar Cognitive Affective Syndrome (CCAS) in this group. We recruited 38 patients with spinocerebellar ataxia type 3 (SCA3) or Machado-Joseph disease (MJD)-SCA3/MJD and 31 controls. Data on disease status were recorded (disease duration, age, age at onset, ataxia severity, and CAG repeat length). the severity of cerebellar symptoms was measured using the International Cooperative Ataxia Rating Scale and the Scale for the Assessment and Rating of Ataxia. the neuropsychological assessment consisted of the Mini-Mental State Examination, Clock Drawing Test, Wechsler Adult Intelligence Scale, Rey-Osterrieth Complex Figure, Wisconsin Card Sorting Test, Stroop Color-Word Test, Trail-Making Test, Verbal Paired Associates, and verbal fluency tests. All subjects were also submitted to the Hamilton Anxiety Scale and Beck Depression Inventory. After controlling for multiple comparisons, spatial span, picture completion, symbol search, Stroop Color-Word Test, phonemic verbal fluency, and Trail-Making Tests A and B were significantly more impaired in patients with SCA3/MJD than in controls. Executive and visuospatial functions are impaired in patients with SCA3/MJD, consistent with the symptoms reported in the CCAS. We speculate on a possible role in visual cortical processing degeneration and executive dysfunction in our patients as a model to explain their main cognitive deficit.
URI: http://repositorio.unifesp.br/handle/11600/34949
ISSN: 1473-4222
Other Identifiers: http://dx.doi.org/10.1007/s12311-011-0318-6
Appears in Collections:Em verificação - Geral

Files in This Item:
There are no files associated with this item.


Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.